Product Name	MAN1B1
Chinese Name	内质网α-甘露糖苷酶1抗体
Alias	Alpha 1 2 mannosidase; Endoplasmic reticulum alpha mannosidase 1; Endoplasmic reticulum mannosyl oligosaccharide 1 2 alpha mannosidase 1; Endoplasmic reticulum mannosyl oligosaccharide 1 2 alpha mannosidase; ER alpha 1 2 mannosidase; Man9GlcNAc2 specific processing alpha mannosidase; MANA ER; Mannosidase alpha class 1B member 1.
Research Area	Cell biology  immunology
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Pig, Horse, )
Applications	ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	80kDa
Cellular localization	cytoplasmic The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human MAN1B1: 211-320/699
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	This gene encodes an enzyme belonging to the glycosyl hydrolase 47 family. This enzyme functions in N-glycan biosynthesis, and is a class I alpha-1,2-mannosidase that specifically converts Man9GlcNAc to Man8GlcNAc isomer B. It is required for N-glycan trimming to Man5-6GlcNAc2 in the endoplasmic-reticulum-associated degradation pathway. Mutations in this gene cause autosomal-recessive intellectual disability. Alternative splicing results in multiple transcript variants. A related pseudogene has been identified on chromosome 11. [provided by RefSeq, Dec 2011] Function: Involved in glycoprotein quality control targeting of misfolded glycoproteins for degradation. It primarily trims a single alpha-1,2-linked mannose residue from Man9GlcNAc2 to produce Man8GlcNAc2, but at high enzyme concentrations, as found in the ER quality control compartment (ERQC), it further trims the carbohydrates to Man5-6GlcNAc2. Subcellular Location: Endoplasmic reticulum membrane; Single-pass type II membrane protein. Tissue Specificity: Widely expressed. DISEASE: Mental retardation, autosomal recessive 15 (MRT15) [MIM:614202]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptive behavior and manifested during the developmental period. Note: The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the glycosyl hydrolase 47 family. SWISS: Q9UKM7 Gene ID: 11253 Database links: Entrez Gene: 11253 Human Entrez Gene: 227619 Mouse Omim: 604346 Human SwissProt: Q9UKM7 Human Unigene: 591887 Human

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